000127763 001__ 127763
000127763 005__ 20240228140947.0
000127763 0247_ $$2doi$$a10.1016/j.ccell.2015.01.002
000127763 0247_ $$2pmid$$apmid:25670083
000127763 0247_ $$2ISSN$$a1535-6108
000127763 0247_ $$2ISSN$$a1878-3686
000127763 0247_ $$2altmetric$$aaltmetric:3442918
000127763 037__ $$aDKFZ-2017-03785
000127763 041__ $$aeng
000127763 082__ $$a610
000127763 1001_ $$aWegert, Jenny$$b0
000127763 245__ $$aMutations in the SIX1/2 pathway and the DROSHA/DGCR8 miRNA microprocessor complex underlie high-risk blastemal type Wilms tumors.
000127763 260__ $$aCambridge, Mass.$$bCell Press$$c2015
000127763 3367_ $$2DRIVER$$aarticle
000127763 3367_ $$2DataCite$$aOutput Types/Journal article
000127763 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1508922845_22424
000127763 3367_ $$2BibTeX$$aARTICLE
000127763 3367_ $$2ORCID$$aJOURNAL_ARTICLE
000127763 3367_ $$00$$2EndNote$$aJournal Article
000127763 520__ $$aBlastemal histology in chemotherapy-treated pediatric Wilms tumors (nephroblastoma) is associated with adverse prognosis. To uncover the underlying tumor biology and find therapeutic leads for this subgroup, we analyzed 58 blastemal type Wilms tumors by exome and transcriptome sequencing and validated our findings in a large replication cohort. Recurrent mutations included a hotspot mutation (Q177R) in the homeo-domain of SIX1 and SIX2 in tumors with high proliferative potential (18.1% of blastemal cases); mutations in the DROSHA/DGCR8 microprocessor genes (18.2% of blastemal cases); mutations in DICER1 and DIS3L2; and alterations in IGF2, MYCN, and TP53, the latter being strongly associated with dismal outcome. DROSHA and DGCR8 mutations strongly altered miRNA expression patterns in tumors, which was functionally validated in cell lines expressing mutant DROSHA.
000127763 536__ $$0G:(DE-HGF)POF3-312$$a312 - Functional and structural genomics (POF3-312)$$cPOF3-312$$fPOF III$$x0
000127763 588__ $$aDataset connected to CrossRef, PubMed,
000127763 650_7 $$2NLM Chemicals$$aDGCR8 protein, human
000127763 650_7 $$2NLM Chemicals$$aHomeodomain Proteins
000127763 650_7 $$2NLM Chemicals$$aMicroRNAs
000127763 650_7 $$2NLM Chemicals$$aNeoplasm Proteins
000127763 650_7 $$2NLM Chemicals$$aNerve Tissue Proteins
000127763 650_7 $$2NLM Chemicals$$aRNA-Binding Proteins
000127763 650_7 $$2NLM Chemicals$$aSIX1 protein, human
000127763 650_7 $$2NLM Chemicals$$aSIX2 protein, human
000127763 650_7 $$0EC 3.1.26.3$$2NLM Chemicals$$aDROSHA protein, human
000127763 650_7 $$0EC 3.1.26.3$$2NLM Chemicals$$aRibonuclease III
000127763 7001_ $$0P:(DE-He78)4096eeffdfc73b75e8ba63dc621a017d$$aIshaque, Naveed$$b1$$eFirst author$$udkfz
000127763 7001_ $$aVardapour, Romina$$b2
000127763 7001_ $$aGeörg, Christina$$b3
000127763 7001_ $$0P:(DE-He78)73316f3cd41c27508dbb6f4c5e7e7a8a$$aGu, Zuguang$$b4$$udkfz
000127763 7001_ $$0P:(DE-He78)e247efc0264a7b0e4bae09804002d4e1$$aBieg, Matthias$$b5$$udkfz
000127763 7001_ $$aZiegler, Barbara$$b6
000127763 7001_ $$aBausenwein, Sabrina$$b7
000127763 7001_ $$aNourkami, Nasenien$$b8
000127763 7001_ $$aLudwig, Nicole$$b9
000127763 7001_ $$aKeller, Andreas$$b10
000127763 7001_ $$aGrimm, Clemens$$b11
000127763 7001_ $$aKneitz, Susanne$$b12
000127763 7001_ $$aWilliams, Richard D$$b13
000127763 7001_ $$aChagtai, Tas$$b14
000127763 7001_ $$aPritchard-Jones, Kathy$$b15
000127763 7001_ $$avan Sluis, Peter$$b16
000127763 7001_ $$aVolckmann, Richard$$b17
000127763 7001_ $$aKoster, Jan$$b18
000127763 7001_ $$aVersteeg, Rogier$$b19
000127763 7001_ $$aAcha, Tomas$$b20
000127763 7001_ $$aO'Sullivan, Maureen J$$b21
000127763 7001_ $$aBode, Peter K$$b22
000127763 7001_ $$aNiggli, Felix$$b23
000127763 7001_ $$aTytgat, Godelieve A$$b24
000127763 7001_ $$avan Tinteren, Harm$$b25
000127763 7001_ $$avan den Heuvel-Eibrink, Marry M$$b26
000127763 7001_ $$aMeese, Eckart$$b27
000127763 7001_ $$aVokuhl, Christian$$b28
000127763 7001_ $$aLeuschner, Ivo$$b29
000127763 7001_ $$aGraf, Norbert$$b30
000127763 7001_ $$0P:(DE-He78)78b6aa82148e60b4d91e3a37a6d3d9a0$$aEils, Roland$$b31$$udkfz
000127763 7001_ $$0P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aPfister, Stefan$$b32$$udkfz
000127763 7001_ $$0P:(DE-He78)4c28e2aade5f44d8eca9dd8e97638ec8$$aKool, Marcel$$b33$$udkfz
000127763 7001_ $$aGessler, Manfred$$b34
000127763 773__ $$0PERI:(DE-600)2074034-7$$a10.1016/j.ccell.2015.01.002$$gVol. 27, no. 2, p. 298 - 311$$n2$$p298 - 311$$tCancer cell$$v27$$x1535-6108$$y2015
000127763 909CO $$ooai:inrepo02.dkfz.de:127763$$pVDB
000127763 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)4096eeffdfc73b75e8ba63dc621a017d$$aDeutsches Krebsforschungszentrum$$b1$$kDKFZ
000127763 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)73316f3cd41c27508dbb6f4c5e7e7a8a$$aDeutsches Krebsforschungszentrum$$b4$$kDKFZ
000127763 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)e247efc0264a7b0e4bae09804002d4e1$$aDeutsches Krebsforschungszentrum$$b5$$kDKFZ
000127763 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)78b6aa82148e60b4d91e3a37a6d3d9a0$$aDeutsches Krebsforschungszentrum$$b31$$kDKFZ
000127763 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)f746aa965c4e1af518b016de3aaff5d9$$aDeutsches Krebsforschungszentrum$$b32$$kDKFZ
000127763 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)4c28e2aade5f44d8eca9dd8e97638ec8$$aDeutsches Krebsforschungszentrum$$b33$$kDKFZ
000127763 9131_ $$0G:(DE-HGF)POF3-312$$1G:(DE-HGF)POF3-310$$2G:(DE-HGF)POF3-300$$3G:(DE-HGF)POF3$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lKrebsforschung$$vFunctional and structural genomics$$x0
000127763 9141_ $$y2015
000127763 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bCANCER CELL : 2015
000127763 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS
000127763 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline
000127763 915__ $$0StatID:(DE-HGF)0310$$2StatID$$aDBCoverage$$bNCBI Molecular Biology Database
000127763 915__ $$0StatID:(DE-HGF)0600$$2StatID$$aDBCoverage$$bEbsco Academic Search
000127763 915__ $$0StatID:(DE-HGF)0030$$2StatID$$aPeer Review$$bASC
000127763 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bThomson Reuters Master Journal List
000127763 915__ $$0StatID:(DE-HGF)0110$$2StatID$$aWoS$$bScience Citation Index
000127763 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection
000127763 915__ $$0StatID:(DE-HGF)0111$$2StatID$$aWoS$$bScience Citation Index Expanded
000127763 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine
000127763 915__ $$0StatID:(DE-HGF)1030$$2StatID$$aDBCoverage$$bCurrent Contents - Life Sciences
000127763 915__ $$0StatID:(DE-HGF)1050$$2StatID$$aDBCoverage$$bBIOSIS Previews
000127763 915__ $$0StatID:(DE-HGF)9920$$2StatID$$aIF >= 20$$bCANCER CELL : 2015
000127763 9201_ $$0I:(DE-He78)B080-20160331$$kB080$$lTheoretische Bioinformatik$$x0
000127763 9201_ $$0I:(DE-He78)B062-20160331$$kB062$$lPädiatrische Neuroonkologie$$x1
000127763 980__ $$ajournal
000127763 980__ $$aVDB
000127763 980__ $$aI:(DE-He78)B080-20160331
000127763 980__ $$aI:(DE-He78)B062-20160331
000127763 980__ $$aUNRESTRICTED